Differential Diagnosis of Disc Edema

Moderated by: Dr. Parul Ichhpujani

Presented by: Dr. Sahil Thakur

Historical Context

1860: Albrecht von Graefe first reported his observations in 4patients with brain tumor and a swelling of the ONH; hecalled it Stauungspapille.

1908:Parsons coined the English term papilledema

1911: Paton and Holmes differentiated between papilledemawith increased intracranial pressure (ICP) and optic neuritis.

Basic differentiation was based on visual function.

Edema of the nerve head with decreased vision was considered opticneuritis and edema of the ONH without decreased vision waspapilledema secondary to increased ICP.

As experience has taught us, decreased vision also occurs as a resultof chronic papilledema secondary to increased ICP and subsequentoptic nerve atrophy.

Pathogenesis

Weiss and Hiscoe’s concept:

Axoplasmic flow: Flow of subcellular and molecular particles along the axonsof the nerves

3 types of axoplasmic flow exist; these occur simultaneously and somewhatindependently and are affected by different pathologic processes.

Orthograde rapid flow, which moves along the axon at a rate of 200 to 1000mm/day and subserves synaptic transmission.

Orthograde slow flow, which moves at 0.5 to 3 mm/day. Maintains the growthand metabolic stability of the axon.

Retrograde flow that moves at 50 to 75 mm/day. Allows the axon to sample itsenvironment and send information back to the cell body.

Different mechanisms of insult differentially affect the axoplasmic flowcomponents.

Ischemia predominantly blocks rapid axoplasmic flow, whereas compression ofan axon blocks slow axoplasmic flow.

The former situation occurs in anterior ischemic optic neuropathy, and thelatter in increased intracranial pressure.

Causes of Disc Edema

Most common causes of optic nerve swelling are: Non-arteritic anterior ischaemic optic neuropathy (35%) Optic neuritis (31%) Intracranial pathology (14%)

Causes of Unilateral optic disc swelling: Demyelinating optic neuritis NA-AION Retinal vein occlusion Diabetic papillopathy

Causes of Bilateral optic disc swelling: Papilledema, Toxic optic neuropathy Malignant hypertension PIH

Jung JJ, Baek S-H, Kim US. Korean Journal of Ophthalmology : KJO. 2011;25(1):33-36.

Differential Diagnosis ofDisc Edema

Diagnostic and Prognostic Clues from Onset of vision loss

Symptoms(Ocular) Transient visual obscuration (TVO)

Central vision affected late

Horizontal diplopia

Decreased Visual Acuity

Blind spot enlargement

Blind spot enlargement and fundus findings in bilateral disc edema

Features of raised ICT/mass lesions like:

Headache more in the morning, intensifies with head movement, coughing or straining.

Projectile vomiting. Loss of consciousness/ generalized motor rigidity.

Symptoms(Systemic)

Signs of Disc Edema

Mechanical signs

Elevation of the optic disc (3D=1mm)

Blurring of the optic disc margins

Filling in of optic cup

Edema of peripapillary nerve fiber

Retinal or choroidal folds (Patton Lines)

Diagnosis is done best by binocular stereoscopic viewing using ahigh convex lens with magnification (90D)

Signs of Disc Edema

Vascular signs

Hyperemia of disc

Venous congestion

Peripapillary hemorrhages

Exudates in disc or peripapillary area

Nerve fiber layer infarcts

Absence of SVP

Mechanism of Vision Loss

• Retinal displacement and retinal elevation of percipient elements and the Stiles-Crawford effect are three of the more traditional explanations for vision loss.

• A report suggesting a relative scotoma on the basis of induced hyperopia has beensuggested by Corbett et al. The hypothesis is that edematous elevations of thepercipient elements away from the blood supply of the choriocapillaris results in adecrease in sensitivity.

Corbett JJ, Jacobson DM, Mauer RC, Thompson HS.Am J Ophthalmol 105:261–265, 1988

I. Papilledema

Differential Diagnosis of Disc Edema

Frisen Papilledema Grading System

Grade 0

Mild nasal NFL elevation.

Rare obscuration of a portion of major vessel (usually at superior pole)

Frisen L. Swelling of the optic nerve head: A staging scheme. J Neurol Neurosurg Psychiatry 1982; 45:13-18

Grade 1

Obscuration of nasal border of disc

Normal temporal disc margin

Grade 2Early Papilledema

Obscuration of all the disc borders

Elevation of nasal border

No major vessel obscuration

Grade 3ModeratePapilledema

Obscuration of all borders

Increased diameter of optic nerve head

Obscuration of segment of major blood vessels as they pass disc margin.

Grade 4MarkedPapilledema

Elevation of entire nerve head

Obscuration of all the borders

A segment of major vessel obscured on the disc

Grade 5Severe Papilledema

Anterior extension of optic nerve head

Total obscuration of vessel on disc surface

Obliteration of optic cup

Papilledemaor Papillitis?

Once true disc edema is established, papilledema(due to raised ICT) has to be distinguished fromother optic neuropathies which can be of variedetiology

Main difference: Visual acuity and optic nerve function Papilledema: Normal

Papillitis: Disturbed

Papilledema Papillitis(Optic Neuritis)

Laterality Bilateral Unilateral

Symptoms Transient obscuration of vision Sudden diminution of vision

Extra ocular Movements No pain Pain on extra ocular movement

Pupillary Reaction Normal RAPD

Media Clear Posterior vitreous cells

Disc elevation 2-6 D Does not exceed 2-3D

Venous engorgement, peripapillary hemorrhages

More frequent Less frequent

Papilledema

Check BP

Stage IV Hypertensive Retinopathy

Bilateral disc edema, other signs

of raised ICT

II. Grade IV Hypertensive Retinopathy

Malignant hypertension

Young individuals, smokers and uncontrolled blood pressure

Severe attenuation of arterioles

Neuroretinopathy, presence of disc edema, multiple cotton wool patches, hard exudates, macular star

Poor prognosis associated with renal insufficiency, stroke, myocardial infarction and death

Henderson AD, et al. Western Journal of Emergency Medicine. 2012;13(6):529-534.

• Benign unilateral or bilateral optic neuropathy with transient optic diskedema and minimal reduction in visual function.

• Disc edema typically resolves in a few months with no resulting opticatrophy and minimal or no decrease in acuity.

III. Diabetic Pappillopathy

• Occurs mainly in younger diabetics• Mild painless visual impairment

that is unilateral in more than half of cases; bilateral disc swelling mandates the exclusion of raised intracranial pressure.

• Hyperaemic disc swelling is characteristic, and disc telangiectasia occasionally mistaken for neovascularization is present in many affected eyes

• Resolution occurs over several months, often leaving mild disc pallor.

Management:• Good glycemic control.• Anti VEGF’s effective in resolution.• No role of PRP

Neuroimaging -CT scan-MRI

Abnormal

1. Space occupying lesions like tumors,abscesses, hemorrhages, infarcts, AVmalformations

2. Trauma3. Inflammatory4. Extra cranial lesions

1. Idiopathic intracranial hypertension

2. Cerebral venous thrombosis3. Endocrinal abnormalities4. Drug overdose/ withdrawal5. SLE

Normal

Normal BP

IV. IIH

• Idiopathic intracranial hypertension is characterized by signs and symptoms of raised intracranial pressure (ICP) with no established pathogenesis.

• The disorder is strongly associated with obesity, and patients are mostly female and typically of reproductive age.

Modified Dandy criteriafor IIH

Papilledema

Check BP Hypertensive retinopathy

Neuroimaging

Abnormal Normal Intracranial space occupying lesions

Lumbar puncture

Opening pressure high

Idiopathic intracranial hypertension

Normal opening pressure

Abnormal spinal fluid analysis

Meningitis

Characteristics of optic neuropathies

Optic neuropathies should be considered under two circumstances

Visual loss associated with anomalous, swollen or pale disc

Fundus is normal, but acuity, color vision, field abnormalities are accompanied by RAPD

Other optic neuropathies causing Disc Edema

Anterior optic neuropathyInflammatory optic neuropathy

Ischemic optic neuropathy

Compressive optic neuropathy

Toxic and hereditary optic neuropathy

Infiltrative optic neuropathy

Intraocular causes CRVO, posterior uveitis, posterior scleritis, hypotony

Neuroretinitis

V. Optic neuritis

It is defined as inflammation of the optic nerve head associated with decrease in visual acuity or visual field loss.

Additional features

Multiple sclerosis

Pain and tenderness

Central and centrocecalscotoma

Contrast sensitivity

MRI:periventricular plaques

Typical optic neuritis

Young adult

Usually associated with multiple sclerosis

Vision starts to improve by 2-3 weeks

MRI is the only required investigation in typical optic neuritis

Atypical optic neuritis

Marked disc swelling

Vitritis

Progression of visual loss after 1 week

Lack of partial recovery within 4 weeks of onset

Persistent pain, no response to steroids

Requires: MRI CSF cytology Syphilis:

MHATP Lyme Titre Sarcoidosis:

CXR, ACE Lupus:ANA Nutritional:B12

Pérez Bartolomé F, et al. (2015) Diagnosis Approach of Optic Neuritis. J Neurol Neurophysiol 6: 345.

VI. Ischemic optic neuropathy

Sudden loss of vision

Interference with blood supply of the posteriorciliary artery to the anterior part of the opticnerve

Can be arteritic or non arteritic:

Arteritic: Associated with Giant cell arteritis. Ophthalmic emergency

Non arteritic: No overt symptoms, associated with hypertension, DM, hypercholesterolemia and shock.

Arteritic Non arteritic

Sex predilection Females>males Females=males

Age >60 years 40-60 years

Visual loss Severe Moderate, on awakening

Associated symptoms Pain, jaw claudication, headache, bright light amarousis

No pain

Second eye involvement Within days or weeks(70%) In months (30-40%)

Disc Pallor> hyperemia, chalky white Hyperemic > pallorSectoral edema

ESR >90mm/hr <40mm/hr

Arteritic Non arteritic

Other signs of ocular ischemia May be present Not present

Anatomic predisposition None Small crowded disc

Late optic atrophy Can have cupping Simple pallor

Response to steroids Vision-sometimesSystemically-definite

None

Fluorescein angiography Choroidal filling defects Normal, can have delayed optic nerve head filling

Arteritic anterior ischaemic optic neuropathy (AAION) is caused by giant cell arteritis (GCA). About 50% of patients with GCA have polymyalgia rheumatica (PMR) at diagnosis, while around 20% of PMR patients will develop GCA. PMR is characterized by pain and stiffness in proximal muscle groups, typically the shoulders and biceps, that is worse on waking

AION

NAIONDisc filling defects

Sectoral edema

Chalky white disc

Disc edema

VII. Compressive optic neuropathy

Disc appearance Disc swelling

Opticociliary shunts

Foster Kennedy syndrome

Additional features

Eg: Optic nerve gliomas Glioblastomas

Meningiomas

Aneurysms

Slowly progressive visual loss

Proptosis, gaze evoked amarousis

Pseudo-Foster Kennedy Syndrome: Unilateral optic disc swelling with contra lateral optic atrophy in the absence of an intracranial mass causing compression of the optic nerve. Occurs typically due to bilateral sequential optic neuritis or ischemic optic neuropathy.

Foster–Kennedy syndrome refers to a constellation of findings associated with tumors of the frontal lobe.

• Optic atrophy in the ipsilateral eye• Disc edema in the contralateral eye• Central scotoma (loss of vision in the middle of the visual

fields) in the ipsilateral eye• Anosmia (loss of smell) ipsilaterally

Bhatnagar KR, Raulji C, Kumar P, Solanki D. Med J DY Patil Univ2014;7:385-7

Natalia Pastora-Salvador, Jesus Peralta-Calvo.CMAJ December 13, 2011 vol. 183 no. 18.

VIII. Hereditary optic neuropathy

Disc appearance

Disc hyperemia

Obscuration of disc margins

Dilated capillaries on disc surface that may extend into surrounding retina (telangiectatic microaneurysms),

Additional features

Swelling of NFL layer and dilatation

Tortuosity of posterior pole vasculature

Maternally inherited mt DNA mutations

Males, 15-35 years

Subacute painless severe loss of vision in one eye, followed by the other

Leber HereditaryOptic Neuropathy A. Acute Stage of LHON

B. Marked Telangiectatic Microangiopathy

C. Late Atrophic appearance

IX. Toxic optic neuropathy

Optic neuropathy due to methanol poisoning is different from others as it causes sudden visual loss and disc edema.

Disc edema is indistinguishable from papilledema

Other symptoms are headache, dyspnoea, vomiting, abdominal pain and bilateral visual blurring.

Sharma P, Sharma R. Toxic optic neuropathy. Indian J Ophthalmol 2011;59:137-41

X. Neuroretinitis

Leber’s stellate neuroretinitis

No risk of MS

Cat scratch disease, syphilis, Lyme disease, HIV

Look for systemic cause

Presents like Optic Neuritis

Good prognosis responds to steroids

XI. CRVO

Disc appearance Hyperemic edematous disc

Neovascularization

Additional features Retinal hemorrhages in all

four quadrants

Dilated, tortuous veins in all four quadrants

Macular edema

Decreased Acuity

RAPD

Disc edema in children

Pediatric papilledema

Infrequent in infants

In children, most common cause is neoplasms

Craniosynostosis

Child abuse, shaken baby syndrome, battered baby syndrome-look for retinal hemorrhages.

Papilledema indicates subdural hematoma

Pediatric optic neuritis

Usually bilateral, disc swelling more common

More aggressive treatment

Immune mediated Usually bilateral, post infectious

Acute demyelinating encephalopathy

Good prognosis

Idiopathic Demyelination

10-50% eventually develop MS

Is there true disc edema…?

Causes of pseudo disc edemaOptic nerve head drusen : disc elevation

Medullated nerve fibres : blurred margins

Morning glory syndrome: elevated disc

Tilted disc: blurred margins

Small hyperopic disc: hyperemic disc

Optic disc dysplasia

Bergmeister’s papilla

True disc edema Pseudo disc edema

Disc color Hyperemic Yellow

Nerve fibre layer Opacified Transparent

Large vessels Normal Anomalous- trifurcation, spoke like

Spontaneous venous pulsation Absent Present in 80%

Hyaline bodies Absent May be present

Optic cup Normal initially, filled Small or absent

Nerve fibre layer hemorrhages Frequent Absent

Fluorescein angiography Dye leakage at disc No leakage/ late staining

Role of investigations in disc edema

1. Visual fields

Papilledema, perineuritis: enlarged blind spot, nasal arcuate scotomas

AION: altitudinal defects

Optic neuritis, toxic optic neuropathies: central scotoma, centrocecal scotoma

Tilted disc syndrome: bitemporal hemianopia which does not respect the vertical midline

2. Fundus fluorescein angiography

Papilledema: disc capillary dilatation, dye leakage and microaneurysm formation

AAION: delayed filling in choroidal phase

NAION: delayed disc filling, segmental disc fluorescence (surface telangiectasias)

Neuroretinitis: no leakage at macula

Hypertensive retinopathy: leakage from small vessels at macula

NAIONStage IV HTNR

3. Neuroimaging

6. Role of USG in Differential Diagnosis of

Disc Edema

Optic Disc Drusen

Management

ONTT Based on the findings of the ONTT, the study group made several recommendations:

• Chest x-ray, blood tests and lumbar puncture are not indicated for typical cases of optic neuritis

• Treatment with oral prednisolone in conventional doses alone, is contraindicated• Consider treatment with intravenous steroids when 3 or more signal abnormalities

are present on MRI to reduce 2-year risk of developing MS, or in patients requiring expedited recovery of vision (i.e., monocular patients, employment demands, bilateral involvement and patients desiring intervention)

Beck RW, Trobe JD, What we have learned from the Optic Neuritis Treatment Trial, Ophthalmology. 1995 Oct;102(10):1504-8.

Management1.AAION2.NAION

NAION Treatment:• There is no definitive treatment.• Optic nerve fenestration has not been shown to be ofbenefit.• Some authorities advocate short-term systemic steroidtreatment.• Any underlying systemic predispositions should be treated.• Although aspirin is effective in reducing systemic vascularevents and is frequently prescribed in patients with NAION,it does not appear to reduce the risk of involvement of thefellow eye.

AAION Treatment:• Absence of visual symptoms: oral prednisolone

1 mg/kg/day • CRP may play an important role in monitoring

disease activity• Treatment is aimed at preventing blindness of

the fellow eye• The second eye may still become involved in

25% despite early steroid administration. • Intravenous methylprednisolone, 500 mg to 1

g/day for 3 days followed by oral prednisolone 1–2 mg/kg/day. After 3 more days the oral dose is reduced to 50–60 mg (not less than 0.75 mg/kg) for 4 weeks or until symptom resolution and ESR/CRP normalization

• Antiplatelet therapy, e.g. aspirin 600 mg stat then 100 mg/day should be commenced as this has been .shown to reduce the risk of visual loss and stroke.

• Immunosuppressives such as methotrexate may be used as adjuncts or as steroid-sparing agents.

Medical treatment options for IIH:• Lifestyle and diet alterations• Corticosteroid therapy

Papilledema in PIH

General : Bed rest.

Control of BP.

Control of Edema : Diuretic, Hypertonic glucose.

Non responders : Termination of pregnancy.

Schiffman JS, Scherokman B, Tang RA, Dorotheo EU, Prieto P, Varon J, Evaluation and treatment of papilledema in pregnancy.,Compr Ophthalmol Update. 2006 Jul-Aug;7(4):187-202.

Surgical Decompression

Indications:

Failure of Medical treatment- Marked disc swelling(>5D)- Engorged veins- Extensive haemorrhages- Early exudate spots- Progressive headache

Progressive optic neuropathy (early field constriction)

Surgical options:• Optic nerve sheath decompression• Bariatric surgery• Frequent lumbar punctures • Sub temporal decompression• Placement of Lumbo-peritoneal shunts.